Isolated primary Langerhans' cell histiocytosis of central nervous system

Solitary eosinophilic granuloma that involves the CNS is an uncommon lesion and most frequently affects the hypothalamus. We report a new and rare case of solitary eosinophilic granuloma of the left temporal lobe in a patient without systemic disease. The diagnosis was confirmed by electron microscopy and immuohistochemical techniques. The treatment of choice is surgical resection. There is a better prognosis in comparison with cases accompanied by systemic disease.     

Epidural hematoma from a cranial eosinophilic granuloma

The formation of an epidural hematoma from an eosinophilic granuloma of the skull is an exceptional occurrence. A 9-year-old boy presented with severe headache, somnolence and vomiting following a minor head injury. Cranial computerized tomography scan showed a seemingly depressed skull fracture together with an epidural hematoma in evolution. A neoplasm and an epidural hematoma were removed at operation. Histopathological study of the excised mass confirmed the diagnosis of eosinophilic granuloma.     

A case of calvarial eosinophilic granuloma with rapid expansion and wide skull invasion: immunohistochemical analysis of Ki-67.

A 15-year-old male visited our neurological service with progressive occipitalgia. Computed tomography showed an osteolytic lesion at the occipital cranium, which grew rapidly during a month. Magnetic resonance imaging showed the lesion as low intensity on T1- and high intensity on T2-weighted images. The mass was soft and successfully removed with surrounding cranium. Histological examination revealed an eosinophilic granuloma with wide skull invasion. Immunohistochemical analysis on the specimen disclosed Ki-67 expression with labeling index of 6.2%. Relatively high proliferative activity in the present case indicates that the local replication of Langerhans' cells as well as inflammatory response contributes to the expansion of calvarial eosinophilic granuloma. This is the first report demonstrating the result of Ki-67 expression on calvarial eosinophilic granuloma.     

纳米羟基磷灰石/聚酰胺66人工椎体置入与椎体重建

摘要 背景：发生于胸腰椎的骨嗜酸性肉芽肿临床较少见,对于病灶切除后的椎体缺损,已有不同生物材料的椎体替代物用于临床,但是纳米羟基磷灰石/聚酰胺66人工椎体在此病的临床应用还鲜有报道。 目的：观察纳米羟基磷灰石/聚酰胺66人工椎体在脊椎嗜酸性肉芽肿病椎切除术后椎体重建的效果。 方法：回顾性分析2005-01/2009-12重庆医科大学附属第一医院骨科收治的经病理证实的胸腰椎嗜酸性肉芽肿患者15例,全部行前路病灶切除、椎体间行纳米羟基磷灰石/聚酰胺66人工椎体置入、前路钉板系统内固定治疗。观察切口愈合情况、伤口并发症及脊髓功能恢复情况。 结果与结论：15例患者全部获得随访,平均随访2.1年。全部痊愈,脊髓功能得到恢复,无复发及并发症发生。提示脊椎嗜酸性肉芽肿病椎切除后椎体间纳米羟基磷灰石/聚酰胺66人工椎体置入、前路钉板系统内固定可以获得良好的椎体重建效果。 关键词：纳米羟基磷灰石/聚酰胺66;人工椎体;胸腰椎;嗜酸性肉芽肿;椎体重建 doi:10.3969/j.issn.1673-8225.2010.47.037     

Solitary Langerhans cell histiocytosis orbital lesion: case report and review of the literature

Solitary eosinophilic granuloma is a rather benign and localized form of Langerhans's cell histiocytosis. Definitive diagnosis is made by histopathology including immunohistochemical detection of S-100, HLA-DR and CD1a antigens. We report the case of a twenty-five year old boy presented with headache and orbit's pain. A CT scan showed a left supero-lateral orbital mass with evidence of bone erosion. The different options of treatment are discussed and the literature is reviewed.     

Moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma

Moyamoya disease has been associated with renal artery stenosis, cerebral hemorrhage, and multiple cranial traumas. We report a unique case of moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma. Although the etiology of moyamoya disease is unknown, a familial pattern of occurrence has been documented. Of particular importance is its presentation with polycystic kidney disease, an autosomal dominant disease, suggesting a hereditary component to the etiology of this unusual vasculitic disease.     

Eosinophilic granuloma of the cervical spine with atypical neurological, cerebrospinal fluid and radiological manifestations]

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteriods (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.     

Eosinophilic granuloma of C4 causing spinal cord compression

A rare case of vertebral eosinophilic granuloma (C4) causing spinal cord compression is reported. The clinical, histological and radiological features of this pathological entity are discussed. After surgery a complete neurological recovery was observed. The value, in selected cases, of surgical treatment with total removal of the tumour and reconstruction of the spine to ensure spinal stability and to prevent irreversible neurological deficit is emphasized.     

Skull fracture mimicking eosinophilic granuloma

Background

Delayed swelling after skull fractures is an uncommon complication following head trauma in children. Classically, growing skull fractures typically present in patients under 3 years of age with progressive subcutaneous fluid collections, or occasionally with neurologic symptoms. We present the case of a healthy 2-year-old boy with a lytic “punched-out” frontal skull lesion. The child presented 2 months after a minor forehead injury for which no medical attention was sought.

Methods

The skull defect had no associated leptomeningeal cyst or brain herniation. Imaging and presentation were thought to be consistent with eosinophilic granuloma. Histologic findings demonstrated a healing skull fracture.

Results

Cranioplasty was performed, and the patient had an uncomplicated postoperative course.

Conclusions

In this report, we describe our experience with this atypical presentation of a healing skull fracture mimicking a typical eosinophilic granuloma.

     

Eosinophilic Granuloma Presenting as an Epidural Hematoma and Cyst

Langerhans'' cell histiocytosis (LCH) is a rare immunologic disorder characterized by histiocyte proliferation in multiple organ systems. Eosinophilic granuloma, a benign bone lesion, represents a focal form of LCH. We experienced a case of Langerhans'' cell histiocytosis in a patient who presented with intracranial epidural hematoma and cyst on the midline of the frontal skull. A 10-year-old boy presented with a rapidly growing large scalp mass on the midline frontal area after mild head trauma. The scalp mass was painless and immobile. Plain skull x-ray showed a punched-out bone lesion. Computed tomography and magnetic resonance imaging showed a non-enhancing osteolytic lesion presenting with an epidural hematoma and cyst on the midline of the frontal skull. The lesion of the skull was completely resected and the patient''s recovery was uneventful. The acute presentation of a solitary eosinophilic granuloma of skull with an epidural hematoma has been described in only five cases in the literature and we report the first case of LCH presenting as an intracranial epidural hematoma on frontal area.     

Multifocal eosinophilic granuloma presenting as progressive brainstem and cerebellar dysfunction.

A 55 year old woman with multifocal eosinophilic granuloma (MEG) is described. She developed facial numbness and twitching followed by slowly progressive cerebellar symptoms. Two years later polyuria and polydipsia were noted. A CT of the brain showed multifocal enhancing lesions, and MRI showed areas of hyperintensity on T2 weighted studies in the cerebellar peduncles, pons, and midbrain. Radiographs of the skull, pelvis, and long bones were normal, but a 99mTc diphosphonate bone scan and MRI showed bone lesions compatible with granuloma. The diagnosis of MEG was made by bone biopsy. This is believed to be the first case of MEG with such unusual clinical profiles and radiographical findings. Skeletal surveys are indicated for patients with unexplained focal or multifocal inflammatory changes in the cerebellum.     

Primary Eosinophilic Granuloma of Adult Cervical Spine Presenting as a Radiculomyelopathy

We report a case of 29-year-old man diagnosed as a primary eosinophilic granuloma (EG) lesion of the seventh cervical vertebra. He had paresthesia on both arms, and grasping weakness for 10 days. Cervical magnetic resonance image (MRI) showed an enhancing mass with ventral epidural bulging and cord compression on the seventh cervical vertebra. Additionally, we performed spine series MRI, bone scan and positive emission tomography for confirmation of other bone lesions. These studies showed no other pathological lesions. He underwent anterior cervical corpectomy of the seventh cervical vertebra and plate fixation with iliac bone graft. After surgical management, neurological symptoms were much improved. Histopathologic evaluation confirmed the diagnosis of EG. There was no evidence of tumor recurrence at 12 months postoperative cervical MRI follow-up. We reported symptomatic primary EG of cervical spine successfully treated with surgical resection.     

Cerebellar involvement in multifocal eosinophilic granuloma: Demonstration by computerized tomographic scanning

Central nervous system involvement outside the hypothalamus or pituitary in multifocal eosinophilic granuloma (MEG) is unusual. Eleven patients with MEG have been examined with cranial computerized axial tomograms (CT). Four patients with moderate to severe cerebellar dysfunction, 3 of whom had no detectable lesions by other neuroradiological techniques, were found to have cerebellar abnormalities. All of the remaining 7 patients with normal neurological examinations had normal CT scans. Computerized axial tomography is a useful technique in the evaluation of patients with MEG and neurological impairment.     

Eosinophilic granuloma of the occipital bone presenting as intracranial venous hypertension.

Intracranial venous sinus thrombosis is an uncommon cause of pseudotumor cerebri. The diagnosis is often not confirmed on the rationale that treatment will not be altered. We report a case presenting a pseudotumor cerebri where the underlying pathology disclosed dural sinus thrombosis resulting from compression by an eosinophilic granuloma of the occipital bone. Routine CT of the head and Technetium-99m brain scan initially demonstrated neither tumor nor thrombosis. Plain skull x-rays subsequently revealed a lytic lesion of the occiput. When reinvestigated with CT using bone density windows the tumor was revealed. Excision of the tumor and a short course of cobalt therapy was curative. Special techniques in nuclear scanning, CT and MRI designed to improve the sensitivity for diagnosing venous sinus thrombosis are described. This case illustrates the importance of establishing a definitive diagnosis and shows the importance of pre-test consultation between clinicians and radiologists to ensure that specific investigative techniques are properly utilized.     

Solitary eosinophilic granuloma of the pediatric skull and spine

Nine pediatric cases of solitary eosinophilic granuloma (EG) are reported. Six children harbored cranial lesions and three had spinal involvement. Plain radiographs and computed tomography scan were not always sufficient for diagnosis. Isotopic bone scan was useful to rule out multiple lesions. The authors' opinion is that surgical excision is the treatment of choice for cranial lesions, leaving radiotherapy for possible relapse. Management of EG of the spine should start with needle biopsy, followed by radiation therapy, surgery being indicated in selected patients with immediate risk of neural compression. Mean follow-up time was 4.4 years (longer than several reported series). At the time of revision all children were asymptomatic, and only one case presented a local recurrence that was managed by radiotherapy.     

Cerebral cysticercus granuloma associated with a subdural effusion.

The association of a solitary cerebral cysticercus granuloma with a subdural effusion is being reported. The granuloma and the effusion resolved following albendazole therapy. We speculate that the spread of the inflammatory changes around the granuloma to the subdural space could have led to the development of the subdural effusion.     

Relapsing polychondritis with an intracranial granuloma: a case report]

We report a case of relapsing polychondritis (RP) with an intracranial granuloma. A 67-year-old man developed progressive disorientation during the course of RP with left auricular chondritis and episcleritis. He had history of sinusitis and rupture of an aneurysm in middle cerebral artery. Laboratory examinations revealed high erythrocyte sedimentation rate and positive C-reactive protein. Head CT and MRI with contrast enhancement showed a mass adjacent to the falx cerebri and lesions in the frontal skull base. The mass was surrounded by extensive perifocal edema that spread mainly into the frontal white matter on both sides. Histologically, the mass displayed an inflammatory granuloma. By removal of the mass, edema decreased around the granuloma, and his disorientation improved markedly. Surgical findings revealed the granuloma was separated from sinusitis. There are a few reports on RP with an intracranial granuloma.     

Multifocal peripheral neuropathy in eosinophilic fasciitis

Summary Polyneuropathy may complicate eosinophilia associated connective tissue disease. We report a multifocal neuropathy in a patient with eosinophilic fasciitis proven by demonstrating an eosinophilic cellular infiltrate in a fascial biopsy specimen from the forearm. Sural nerve biopsy revealed lymphocytic cuffing of epineural arterioles. Although described in thel-tryptophan-related eosinophilia-myalgia syndrome, peripheral neuropathy with these features has not been previously noted in a patient with eosinophilic fasciitis who had not consumedl-tryptophan.     

Severe episodic headache as the sole presenting ictal event in patients with a solitary cysticercus granuloma

OBJECTIVES: Most patients with a solitary cysticercus granuloma present with seizures and severe episodic headache as the sole presenting symptom is rare in these patients. We report the clinical features, evolution and outcome of the disease in patients with a solitary cysticercus granuloma who had severe headache alone without seizures as the sole presenting event. MATERIAL AND METHODS: This was a prospective study which included all patients who presented with severe episodic headache alone and had a diagnosis of a solitary cysticercus granuloma based on CT imaging and follow-up and were managed in our department between June 1, 1991 and May 31, 1998. RESULTS: Out of nearly 550 patients diagnosed to have a solitary cysticercus granuloma during the period of the study, 15 patients (2.5%) presented with severe episodic headache alone. While 9 patients had 1 episode of headache, 6 patients had 2 to 5 episodes. Patients were managed with conservative symptomatic therapy (13 patients) and albendazole therapy (2 patients). Follow-up scans showed complete or partial resolution of the granuloma in 8 patients and a lesion of the same size in 4 patients. None of the patients with complete resolution of the granuloma reported further episodes of headache. CONCLUSIONS: It is important to recognize this presentation of a solitary cysticercus granuloma as it could be confused clinically with other acute central nervous system illnesses such as subarachnoid haemorrhage and meningitis. In regions endemic for cysticercosis a contrast enhanced CT scan of the brain should be performed in patients presenting with sudden onset of severe headache when a plain scan does not reveal intracranial haemorrhage as only a contrast enhanced scan will reveal the granuloma.     

Carbamazepine-Induced Eosinophilic Colitis

Severe watery diarrhea and eosinophilic colitis induced by carbamazepine (CBZ) has not been described previously. We report the first known case of CBZ-induced watery diarrhea and eosinophilic colitis in a 57-year-old man receiving CBZ for secondarily generalized tonic-clonic seizures that developed after a cerebral infarction.